Dexamethasone induces dysferlin in myoblasts and enhances their myogenic differentiation.

摘要: Glucocorticoids are beneficial in many muscular dystrophies but they ineffective treating dysferlinopathy, a rare dystrophy caused by loss of dysferlin. We sought to understand the molecular basis for this disparity studying effects glucocorticoid on differentiation myoblast cell line, C2C12, and dysferlin-deficient C2C12s. found that pharmacologic doses dexamethasone enhanced myogenic fusion efficiency C2C12s increased induction dysferlin, along with specific transcription factors, sarcolemmal structural proteins. In contrast, C2C12 line demonstrated reduction long myotubes early particular muscle proteins, most notably, myosin heavy chain. Dexamethasone partially reversed defect cells. hypothesize key therapeutic benefit glucocorticoids may be up-regulation dysferlin as an important component glucocorticoid-enhanced differentiation.